British Journal of Ophthalmology, 1985, 69, 482-486

Indications for vitrectomy in congenital retinoschisis

JOEL SCHULMAN,' GHOLAM A PEYMAN,' NORBERT JEDNOCK'
AND BRUCE LARSON'

From the Departments of Ophthalmology, 'University of Illinois at Chicago, Eye and Ear Infirmary, and the
'University of Texas Medical Branch, Galveston, Texas

SUMMARY Two patients with congenital retinoschisis developed rhegmatogenous retinal detach-
ments with either a tractional component or associated vitreous haemorrhage. The second patient.
also had a large, raised schisis cavity under vitreous traction, which obscured visualisation of the
macula. Vitrectomy is indicated for some patients with congenital retinoschisis.

Congenital retinoschisis, a relatively rare ocular linked retinoschisis at age 5, was referred to the
disorder' first described by Haas' in 1898, has also University of Illinois Eye and Ear Infirmary with a
been called congenital vascular veils,' cystic disease retinal detachment in his right eye. He was the
of the retina in children,4 juvenile retinoschisis,3 and product of a normal pregnancy and had no congenital
inherited retinal detachment.6 Usually bilateral,7 the anomalies. Two older brothers also had X-linked
disorder is inherited as an X-linked Mendelian retinoschisis. An ocular examination of his mother
recessive trait affecting males,""' though a few and three of his sisters was unremarkable. No con-
sporadic cases have been reported in females." sanguinity existed between the parents. When the
Retinoschisis has a predilection for the infero- patient was 4 years old, an electroretinogram (ERG)
temporal quadrant7 and rarely extends to the ora (performed with the patient under anaesthesia)
serrata. Large blood vessels frequently course showed an extinguished B wave. The boy's two
through the inner retinal wall, and other vascular brothers had similar ERG changes.
elements may traverse the schisis cavity.' Retinal Ocular examination revealed a corrected vision of
holes in the inner layer are common and usually counting fingers at 6 feet (1*8 m) in the right eye and
appear in multiples.'" 5/200 in the left eye. External examination revealed
Other ocular changes reported in retinoschisis 80 of left esotropia. Biomicroscopy was unremark-
include neovascular glaucoma,'4 optic atrophy,'5 able. Ophthalmoscopy of the left eye revealed a large
and diminished visual acuity'6 secondary to foveal grey intravitreal membrane floating in the inferior
dystrophy. Conway and Welsh" described the pres- vitreous between the inferior arcade and equator.
ence of a haemorrhagic retinal cyst surrounded by The macula was distorted, and inferiorly a retinoschisis
exudate extending into the macula. cavity was observed. A fundus examination of the
Retinal detachment is a serious complication right eye disclosed cystic macular changes. A full-
associated with congenital retinoschisis.'7 1.1 7 18 thickness retinal detachment (Fig. 1) that involved
Surgical treatment in the past has involved conven- the macula and most of the inferior retina extended
tional scleral buckling procedures. The use of from the 4 to 8 o'clock meridians. A small schisis
vitrectomy to remove vitreous haemorrhage and to cavity was noted in the inferior retina near the
repair a retinal detachment with a tractional com- equator. Another area of retinoschisis was present
ponent in patients with X-linked retinoschisis is, to inferior to the temporal arcades. A vitreous veil
the best of our knowledge, described for the first time exerted traction on a retinal fold along the infero-
here. temporal arcade. A partial-thickness retinal tear was
also present below the retinal fold.
Case reports During surgery the partial-thickness retinal tear
inferior to the macula was noted to be under con-
CASE 1 siderable vitreous traction. The vitreous band was
An 11-year-old boy, who was diagnosed as having X- transsected with a bipolar'9 cautery attached to the
Corrcspondencc to Dr Gholam A Pcyman, 1855 W Taylor St, fiberoptic membrane dissector and vitrophage to
Chicago, IL 60612, USA. allow the underlying retina, including the partial-
482
Indicationsfor vitrectomy in congenital retinoschisis
thickness retinal tear, to settle. The inferior
peripheral retina settled during the completion of
the vitrectomy. Peripheral transscleral cryopexy
was applied to the eye for 3600.
Postoperatively the retina remained attached
(Fig. 2). At last examination seven months after
surgery the best corrected visual acuity was 20/200.
CASE 2
A 2-year-old black boy had a red left eye that was
diagnosed as viral conjunctivitis. On follow-up
examination three weeks later the child was found to
have a preretinal haemorrhage in the right eye and a
possible retinal detachment in the left eye.
He was living in a foster home with his 3-year-old
brother and no family history was available. Ocular
examination of his brother was unremarkable.
Five weeks after his initial presentation the boy
was admitted to hospital for an examination under
anaesthesia. The right eye showed stellate cystoid
changes in the macula and extensive retinoschisis in
the inferior and temporal periphery, extending
centrally with abrupt termination at the posterior
pole. The left eye had a highly elevated bullous
483
Fig. 1 Case 1. Composite fundus
photograph showingflat detached
retina inferiorly and in the macula.
schisis cavity inferiorly overhanging and obscuring
visualisation of the macula (Fig. 3). A second area of
schisis was present in the superior temporal area.
The patient was again examined under anaesthesia
six weeks later and underwent drainage of schisis
fluid, vitrectomy to relieve traction on the inferior
schisis cavity, and placement of an encircling silicone
band in the left eye. Stellate cystoid changes were
observed in the left macula after the schisis cavities
were flattened.
He was readmitted for an examination under
anaesthesia 10 months later. Examination of the left
eye revealed a reaccumulation of fluid in the inferior
and temporal schisis cavity, which again covered the
macula. Three months later the child underwent
surgery in the left eye, which involved drainage of
fluid from both schisis cavities, and then scattered
transscleral cryocoagulation was applied to the now
flattened inferior and temporal schisis cavity. Twenty
months after operation the retina remained attached
(Fig. 4).
Nineteen months after the initial admission to
hospital a vitreous haemorrhage (Fig. 5) and an
inferior retinal detachment were discovered in the
484 Joel Schulman, Gholam A Peyman, NorbertJednock and Bruce Larson

Fig. 2 Case 1. Fundus photograph

after vitrectomy and retinal
detachment repair.

right eye during routine examination. After a was drained, and the two retinal tears were treated
vitrectomy, to remove blood from the vitreous cavity with cryopexy and an encircling silicone band, and a 5
two large full-thickness retinal tears were observed. mm radial silicone sponge was placed behind both
Subretinal fluid and fluid in the large schisis cavity retinal tears.

Fig. 3 Case 2. Left eye fundus photograph showing huge Fig. 4 (Case 2. Fundus photograph after vitrectomy, retinal
schisis cavity covering the macular area. Arrowpoints to detachment repair, and cryocoagulation. Note attached
optic disc. retina and typical schisis changes of the macula; arrows
indicate cryocoagulation scar.
Indications for vitrectomy in congenital retinoschisis
Fig. 5 Case2. Fundus photograph of right eye showing
vitreous haemorrhage covering a detached inferior retina.
Postoperatively a cataract developed in the right
eye, which required a lensectomy a year later. At the
time of the cataract surgery the retina and schisis
cavities were noted to be flat (Fig. 6). Measurement
of visual acuity was not possible because of the
patient's youth.
Discussion
Congenital retinoschisis may be progressive or
stationary or may spontaneously regress.'3 The
clinical spectrum of ocular changes varies consider-
ably among affected individuals. Deutman2"' states
that macular retinoschisis occurs in all affected
patients. Foveal retinoschisis in one report was
present in 98% of involved individuals.2' According
to Deutman2" in almost half the affected eyes macular
lesions may represent the only sign of this disorder.
The macular changes in older people may appear
atrophic and pigmented.2224 Peripheral retinoschisis,
characterized by intraretinal splitting involving the
nerve fibre layer,' occurs in approximately 50% of
eyes.26The inner layer is often immobile and elevated
in a concave configuration. An absolute scotoma is
present corresponding to the area of retinoschisis.2s
Two severe vision-threatening complications of
congenital retinoschisis are retinal detachment and
vitreous haemorrhage. ' Retinal detachments are
caused by a combination of inner and outer layer
holes in areas of retinoschisis, or retinal tears created
by vitreous traction in abnormal retina.'3 Vitreous
haemorrhage may result from vitreous traction on
. ;x
detachment repair, and buckling procedure.
retinal vessels coursing through the elevated inner
retinal layer of a schisis cavity.
A variety of vitreous pathology has been observed
in eyes with congenital retinoschisis. All 15 patients
in a series reported by Lisch26 showed varying degrees
of vitreous liquefaction. Vitreous strands were found
in 50% of patients by Bec and associates.27 Kraushar
et al. '" reported a series of 40 patients (77 eyes) with
congenital retinoschisis. Retinal detachment devel-
oped in 22% of these affected eyes. Associated with
retinal detachment were pathological vitreous
changes, including vitreous membranes attached to
and in some instances elevating the equatorial retina.
Vitreous traction present in the posterior pole
resulted in pseudopapilloedema (13% of eyes) and
ectopic macula (6% of eyes). Vitreous haemorrhage
occured in 40% of eyes. The investigators believed
that in some instances vitreous haemorrhage was
caused by traction on retinal blood vessels. Other less
common signs of vitreous traction were tenting of the
485
Fig. 6 Case 2. Same eye as in Fig. 5 after vitrectomy, retinal
inner retinal layer (12%) or blood vessels (4%) and
geographic areas of white without pressure (35%).
The retinas in both our patients and the inferior
schisis cavity overhanging and obscuring visualisa-
tion of the macula were under vitreous traction. The
retinas in both cases were successfully reattached
following vitrectomy and scleral buckling. Vitrec-
tomy in eyes with congenital retinoschisis is indicated
when a significant tractional component is associated
with either retinal detachment, elevation of a schisis
cavity covering the macula, or vitreous haemorrhage
originating from blood vessels located in the inner
486 Joel Schulman, Gholam A Peyman, NorbertJednock and Bruce Larson
retinal layer of a schisis cavity. The tractional changes
may result from proliferative vitreoretinopathy28 com-
plicating a retinal detachment or vitrous pathology
inherent in congenital retinoschisis. Scleral buckling
does not treat the vitreous pathology in these eyes,
though it may relieve to some extent the tractional
component of the retinal detachment. Vitrectomy is
necessary to remove vitreous haemorrhage and to
eliminate vitreous traction on the vessels and the
retina.
Although vitrectomy prevents progression of
vitreous tractional changes, it will not eliminate
re.iccumulation of fluid in the schisis cavity, as was
the case in our second patient.
Drainage of subretinal fluid combined with cryoco-
agulation of the suspected retinal holes creates a
permanent chorioretinal scar, thereby preventing
reaccumulation of fluid in the schisis cavity.
This work was supported in part by core grant 1 P3EY01792 from the
National Eye Institutc, Bcthesda, Md, USA.
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